1/37. Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension.BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. methods: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/37. Non-Hodgkin's lymphoma of the thyroid and adrenal glands.We report a case of non-Hodgkin's lymphoma(NHL) with simultaneous involvement of both thyroid and bilateral adrenal glands. literature review on a computerized search showed that this is an extremely rare condition. The final diagnosis of diffuse large B cell lymphoma was confirmed by biopsies of thyroid gland, enlarged cervical lymph node, and adrenal gland. The significant endocrine dysfunction of the thyroid, adrenal or other endocrine glands was absent in our case. The patient responded dramatically to three cycles of chemotherapy with no complication or endocrine dysfunction and continues to be followed.- - - - - - - - - - ranking = 2keywords = dysfunction (Clic here for more details about this article) |
3/37. Autonomic dysfunction associated with multiple pelvic ganglioneuromas.We describe the case of a 59-year-old male in which several symptoms of autonomic dysfunction resulted associated with the presence of multiple ganglioneuromas in the adrenal glands and in paravertebral spaces. In a 2-year period, the patient developed sexual dysfunctions, micturitional disturbances and severe orthostatic hypotension and was erroneously diagnosed as having primary autonomic failure. However, the examination of all the autonomic functions showed that papillary function and cardiovascular reflexes were normal. CT scan of the abdomen revealed the presence of several small masses in the adrenal glands and along the lumbar sympathetic chain. Cytologic examination of the adrenal glands showed clusters of ganglionlike cells interspersed with schwann cells, leading to the diagnosis of ganglioneuromas. patients with signs and symptoms of autonomic dysfunction need an extensive clinical and laboratory examination of all the autonomic functions in order to exclude secondary causes of the symptomatology.- - - - - - - - - - ranking = 7keywords = dysfunction (Clic here for more details about this article) |
4/37. pheochromocytoma presenting as life-threatening pulmonary edema.Acute cardiogenic pulmonary edema as the first presentation of pheochromocytoma is uncommon and usually rapidly fatal. A 39-yr-old man presented in acute cardiogenic shock with global ventricular dysfunction that required high-dose iv inotrope support and an intraaortic balloon pump assist device. Abdominal imaging to exclude aortic dissection revealed a 6-cm right adrenal mass. Significant myocardial infarction (electrocardiographic changes and elevated cardiac enzymes) contributed to the cardiac decompensation. After withdrawal of inotrope support, 24-h urinary catecholamine levels revealed 2,155 nmol/d (<125) of adrenaline and 7,437 nmol/d (<560) of noradrenaline, confirming a pheochromocytoma. The tumor was successfully removed at laparotomy; however, the patient's course was complicated by a thromboembolic cerebrovascular accident with paraplegia. He recovered cardiac function almost completely within 3 wk of medical therapy alone. Although uncommon, this case highlights the need to consider pheochromocytoma early in the management of unexplained cardiogenic shock.- - - - - - - - - - ranking = 136.17271759972keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
5/37. Primary aldosteronism and malignant adrenocortical neoplasia.Our experience indicates that although adrenal carcinoma is not a common cause of primary aldosteronism, 4 to 5% of patients in a single large series may have a malignant adrenocortical tumor. The magnitude of the hypokalemia and the hyperaldosteronuria tends to be greater in patients with malignant tumors, but these patients cannot be clearly separated from those with benign tumors or hyperplasia on this basis. patients who have malignant tumors may have no chemical evidence of adrenocortical dysfunction other than excessive aldosterone secretion. Finally, a good response to spironolactone for months does not exclude adrenal carcinoma as the cause of primary aldosteronism.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
6/37. Adrenal myelolipoma with translocation (3;21)(q25;p11).Adrenal myelolipoma (ML) is a rare, benign, nonfunctioning tumor-like lesion composed of mature adipose tissue interspersed with bone marrow-like hematopoietic elements in various proportions. It occurs usually in adults and is frequently asymptomatic in about half of cases. The histogenesis of adrenal ML is not clear and this lesion has been found to be associated with endocrine disorders, other adrenal dysfunction and tumors, and hyperstimulation with adrenocorticotropic hormone. Specific chromosomal abnormalities, however, have not been observed in such cases. Herein, we report a typical case of adrenal ML found incidentally in a 26-year-old man. Conventional cytogenetic techniques demonstrated balanced translocation between bands 3q25 and 21p11 in 9 of 20 metaphases analyzed in cultured tumor cells. To the best of our knowledge, this is the first reported case of adrenal ML showing chromosomal abnormality. This finding would indicate that adrenal ML is a bona fide neoplasm and the possibility of derivation from misplaced hematopoietic cells may be alternatively taken into consideration in view of the similar genetic changes in hematopolietic neoplasms.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
7/37. myelolipoma associated with adrenal ganglioneuroma.Adrenal myelolipomas are rare, benign mesenchymal tumors composed of mature adipose tissue and hematopoietic cells in varying proportions. Although the majority of cases occur as isolated adrenal lesions, myelolipomas have been described in association with various adrenal pathologic conditions. These conditions include enzyme deficiencies and hyperplastic and neoplastic lesions of the adrenal cortex, with perhaps endocrine dysfunction as a common feature. ganglioneuroma is a benign tumor of the sympathetic nervous system that rarely produces symptoms of endocrine dysfunction. We report an unusual case of myelolipoma associated with ganglioneuroma of the adrenal medulla. The histogenesis of myelolipoma remains speculative. However, the close proximity to adrenal cortical cells within the stroma of ganglioneuroma suggests that the hormonal microenvironment may have played a role in the development of the myelolipoma.- - - - - - - - - - ranking = 2keywords = dysfunction (Clic here for more details about this article) |
8/37. Cardiac failure and multiple organ dysfunction syndrome in a patient with endocrine adenomatosis.In this case report, we present the successful therapy of severe cardiac failure in pituitary adrenal insufficiency. A previously healthy 56-year-old-man in pituitary coma due to an atypical variant of multiple endocrine adenomatosis (pituitary adenoma and pheochromocytoma) suffered from cardiac failure resistant to catecholamine and standard hydrocortisone therapy. After two bolus injections of dexamethasone (2 x 24 mg) mean arterial pressure and cardiac function dramatically improved, probably due to restoration of permissive effects on catecholamine action and reversal of pathophysiological mechanisms of cardiac failure. We conclude that in patients with severe cardiovascular failure in pituitary coma the administration of potent glucocorticoids may be more effective in reversing cardiovascular failure than standard dosages of hydrocortisone.- - - - - - - - - - ranking = 4keywords = dysfunction (Clic here for more details about this article) |
9/37. Unilateral adrenal tumor, erectile dysfunction and infertility in a patient with 21-hydroxylase deficiency: effects of glucocorticoid treatment and surgery.In untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHDS) the presence of adrenal and testicular tumors had been described; however little is known about the effect of the enzymatic defect on fertility in males. We studied a male adult patient affected by 21OHDS for infertility, after a long period of discontinuation of glucocorticoid therapy and then during resumption of treatment and 8 months after monoadrenalectomy. The initial spermatic count revealed azoospermia and testicular needle aspiration showed a cytological picture consistent with prepuberty. The morphofunctional study revealed a right adrenal mass with reduced uptake at radioscan. Treatment was resumed with onset of impotency, which improved after reduction of the dose of glucocorticoids. The patient was monoadrenalectomised and his spermatic count increased. The patient shows that corticosteroid therapy in 21OHDS should be continued lifelong to avoid adrenal hyperplasia with possible areas of autonomy and to allow regular fertility. Impotence during treatment is probably due to a decrease of excessive adrenal androgens while testicular androgen production is still suppressed.- - - - - - - - - - ranking = 4keywords = dysfunction (Clic here for more details about this article) |
10/37. Combined adrenal adenoma and myelolipoma in a patient with Conn syndrome. Case report.Adrenal myelolipoma is an uncommon benign tumor usually discovered by chance in patients with hypertension, obesity, arteriosclerosis or cancer. The association with adrenocortical endocrine dysfunctions is rare. We report herein an unusual case, the second in the literature to the best of our knowledge, of combined adrenal adenoma and myelolipoma in a patient with Conn syndrome.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
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